Search

Persons who provide nutrition education recognize that various factors influence the eating habits of adolescents. Nutrition knowledge has a positive influence on choosing adequate nutrition. The aim of this study was to evaluate the nutrition knowledge and the average calcium intake in high school students in Mostar and Zagreb and to compare the anthropometric and dietetic parameters between them. On 20 questions about the diet, the average number of correct answers of all subjects was 8, while the students from Zagreb had a significantly higher number of correct answers compared to the students from Mostar. The average calcium intake in all subjects was 1285.55 mg, which is below, but very close to the recommendations for adolescents- 1300 mg/day.

In accordance with Regulation on natural mineral, natural spring and table waters (Official Journal no. 81/13), labelling of natural mineral water with claim "can have a laxative effect" and "can have a diuretic effect", require conditions under which this claims can be used. These conditions must meet certain microbiological and physicochemical parameters. Setting microbiological and physicochemical parameters are based on the standards of the Codex Alimentarius Commission (Codex Stan 108-1981-rev. 1997) for natural mineral water, Croatian Regulation on natural mineral, natural spring and table waters (Official Journal no. 81/13), and data available from the literature. Presence of certain chemical elements or their compounds in certain concentrations in mineral water can cause a laxative or diuretic effect. Based on the available scientific literature, the Working group of Croatian Food Agency for issuing scientific opinion on labeling of natural mineral water „can have a laxative effect" and "can have a diuretic effect", concluded as follows: - researches indicate that the laxative effect was observed at the consumption of mineral water with sulphate proportions above 1000 mg / L - researches indicate that the diuretic effect was observed at the consumption of mineral water with lower proportions of minerals (less than 500 mg / L) - with regard to the perceived adaptation of the human body, increased shares of sulfate, and reduced content of minerals in mineral water, which ultimately induce the lack of occurrence of both of the above mentioned effects, Working Group recommends establishing the Commission within the relevant institutions (Ministry of Agriculture) to review each individual requirements for the use of statements "may have laxative effects" and "may have a diuretic effect"., HR; hr; sbasic@hah.hr

Breakfast, as a first meal of the day has shown to improve health and the cognitive abilities of individuals who consume it, especially when it is composed of high-quality nutrients. The aim of this study was to determine what kind of cereals students eat for breakfast and how this affects their micronutrient intake. The subjects were 84 female undergraduates of the Nutrition studies aged from 18 to 24. Questionnaire was used to determine usual breakfast pattern. The results show that all subject consume breakfast at least 2 days a week, yet only 54 % of them eat breakfast every day. Usually, 56 % of subjects eat breakfast cereals (ready-to-eat or cooked cereals), 21 % eat whole wheat bread, 15 % eat white bread and 4 % eat pastry. Average energy intake from breakfast was 395 ± 157 kcal. Subjects who eat breakfast cereals (ready-to-eat or cooked) had higher dietary fiber, thiamine, folate, calcium and iron intake than those who eat bread or pastry (p

S-adenosylhomocysteine hydrolase deficiency: pathogenesis of muscle involvement

Dijagnostički izazovi i liječenje nasljednih bolesti stvaranja energije u mitohondrijima

High plasma chitotriosidase activity in an infant with rapidly progressive Wolman’s disease and novel mutation in LIPA gene

Zadaća medicinskih sestara u zbrinjavanju pacijenata s nedostatkom aktivnosti S-adenozilhomocistein hidrolaze

Posljednjih se godina sve više piše o učestalosti poremećaja vezanih uz funkcionalni manjak vitamina B12 među djecom, mogućnostima dijagnostike i potrebe za što ranijim početkom liječenja. Otkrivene su nove spoznaje vezane uz metabolizam kobalamina, od trenutka unošenja hranom sve do stvaranja njegovih aktivnih oblika unutar stanice: adenozilkobalamina i metilkobalamina. Otkriveni su geni odgovorni za nastanak rijetkih, ali po život opasnih poremećaja vezanih uz unutarstanični metabolizam kobalamina. Vitamin B12 je organizmu neophodan za normalan razvoj. Unosimo ga u organizam hranom. Vezan za bjelančevine hrane njegov metabolizam počinje već u ustima te se dalje putem želuca, vezan za bjelančevinu haptokorin, prenosi do tankog crijeva gdje se veže za intrizični faktor. Putem receptora na terminalnom ileumu kobalamin se procesom proteolize oslobađa u slobodnom obliku u cirkulaciju. Tu se veže za transkobalamin i tako prenosi do svih stanica u tijelu. Unutarstanični metabolizam kobalamina obuhvaća devet stepenica do stvaranja aktivnih oblika vitamina B12 koji su odgovorni za nastanak kliničke slike kod njegovog manjka. Metilkobalamin služi kao supstrat i donor metilne skupine te je kofaktor metionin sintetazi u reakciji u kojoj se homocistein pretvara u metionin i istovremeno 5-metil-tetrahidrofolat u tetrahidrofolat. Te dvije reakcije su neophodne za sintezu DNK i brojne metilacijske reakcije u organizmu (npr. za sintezu mijelina). Adenozilkobalamin služi kao kofaktor metilmalonil- CoA mutazi u reakciji u kojoj se metilmalonil-CoA pretvara u sukcinil-CoA koja je dio Krebsovog ciklusa putem kojeg se stvara energija. U svakome od tih koraka (smanjeni unos hranom, povećane potrebe, poremećaji apsorpcije, transporta ili unutarstaničnog metabolizma kobalamina) može doći do poremećaja u metabolizmu koji se očituju kliničkom slikom manjka jednoga ili oba aktivna oblika kobalamina. Nutritivni manjak je najčešći uzrok manjka kobalamina te se najčešće javlja u dojene djece. U 20% trudnoća je uočen manjak kobalamina u trudnica. Manjak vitamina B12 predstavlja neovisan faktor rizika za anomalije razvoja neuralne cijevi u našoj populaciji, a i u svijetu. Suočeni s tim činjenicama svjesni smo da se u našoj zemlji njegov manjak rijetko prepoznaje, a posljedice koje ostavlja mogu biti trajne. Simptomi koji upućuju na manjak kobalamina su nenapredovanje u tjelesnoj masi, širok spektar neuroloških poremećaja uključujući i konvulzije, te hematološki poremećaji (npr. megaloblastična anemija). Kod sumnje na manjak kobalamina osim koncentracije kobalamina, treba izmjeriti koncentraciju homocisteina, analizirati kompletnu krvnu sliku i odrediti izlučivanje organskih kiselina u urinu. Povišena koncentracija homocisteina i/ili prisutna metilmalonska kiselina u urinu su nam osjetljivi parametri koji upućuju na manjak kobalamina. Liječenje se provodi hidroksikobalaminom u obliku intramuskularnih injekcija.

Enzimsko nadomjesno liječenje bolesnika s mukopolisaharidozom tipa II

Antenatalni Bartterov sindrom u sestre i brata

Enzimska nadomjesna terapija- jedno načelo, brojne teškoće, različiti ishodi

Our patient is the second child of young unrelated parents. Family history is unremarkable. He was born at term after uneventful pregnancy. During the neonatal period horizontal nystagmus and microphtalmia of the right eye were noticed. Ophtalmological examination of the right eye revealed retinal detachment, vitreous organisation and microphtalmia, while on the left eye there was retinal rarefaction with focal proliferation of pigment epithelium and opacities in anterior vitreous. At the age of 2.5 months more thorough clinical evaluation was performed. The child had truncal hypotonia, lower limb hypertonus, reduced spontaneous movements, flexion contractures of hips and knees, delay in neuromotor development and dysmorphic features (dolychochephaly, high palate, depressed nasal bridge, low set ears and poorly shaped right ear). Deep tendon reflexes on lower limbs couldn’ t be elicited. Interesting distinction which we couldn’ t connect to other features was xerosis with regional ichthyosiform skin changes. Biochemical investigation showed permanently very high creatine kinase (the highest value was 9366 U/L). Syalotransferin isoelectrofocusing was normal. Results of pathohystological analysis and electromyoneurography pointed to muscular dystrophy. Brain MR revealed cerebellar cortical dysplasia and subcortical cerebellar cysts, hypoplasia of vermis and pons, polymicrogyria (most pronounced in the frontal region) with granular and irregular cortex – findings typical for MEB. Visual evoked potentials with electroretinography detected severe neuronal impartment of left visual pathway and no response on the right side. At the age of 15 months (last clinical evaluation) child presented with growth retardation, truncal and upper limb girdle hypotonia and severe psychomotor delay (without head control, unable to sit, with reduced spontaneous movements and relatively good social contact). Skin changes remaind as previously. Due to typical clinical presentation (with the exception of skin changes), structural brain abnormalities and course of the disease we thought that child suffered from muscle-eye-brain disease. Unexpectedly, molecular analysis of POMGnT1 and FKRP genes didn’ t reveal mutations. Could this child suffer from some other form of congenital glycosylation disorder? We consider further diagnostic procedures.

The aim of this work was to compare the way of weight control, dietary knowledge related to dietary fibre and cereals and intake of this food, between females which were dieting and non dieters group. The survey population included 253 female students with a mean age and standard deviation (SD) of 22.25± 1.47y. Dieting practices, nutritional knowledge and consumption of products from cereal group were assessed by using a specially designed survey instruments. The questionnaire consisted of demographic questions, questions regarding recent dieting practices, nutritional knowledge questionnaire and food frequency questionnaire. During the survey 34.39% of participants reported that they were trying to lose weight during the previous year. Surprisingly, high percentage (83.92%) of study participants in the acceptable weight category according to BMI, reported dieting, indicating large proportion of normal weight females with weight dissatisfaction. Between dieters only 10.30% were actually overweight (BMI>25). Of those who have tried dieting, 35% used meal skipping to control their weight with concerning proportion (approximately two thirds) of those who usually skip breakfast while the rest regularly skip the evening meal. Limiting portion size was practiced by 21.83% of those who had tried dieting techniques. The increasing frequency of exercise as weight loss method was reported by 16.09% of participants. The tendency to eat less bread was remarked among dieters (42.52% of dieters eat breads daily in comparison with 64.46% of non dieters). Dieters ate more frequently breakfast cereals and had more positive attitude toward whole grain products. Besides better knowledge about positive health effect of fibre consumption, more dieters had correctly identified fibre-rich food.

This paper reports the third proven human case of deficient S-adenosylhomocysteine (AdoHcy) hydrolase activity. The patient is similar to the only two previously reported cases with this disorder in having severe myopathy, developmental delay, elevated serum creatine kinase (CK) concentrations, and hypermethioninaemia. Although he has been followed from infancy, the basic enzyme deficiency was established only at age 26 years. The diagnosis was based on markedly elevated plasma concentrations of both AdoHcy and S-adenosylmethionine, some 20% of the mean control activity of AdoHcy hydrolase activity in haemolysates of his red-blood cells, and two missense mutations in his gene encoding AdoHcy hydrolase. He had low values of erythrocyte phosphatidylcholine and plasma free choline and marginally elevated excretion of guanidinoacetate, suggesting that the elevated AdoHcy may have been inhibiting methylation of phosphatidylethanolamine and guanidinoacetate. His leukocyte DNA was globally more methylated than the DNA's of his parents or the mean extent of methylation measured in age-matched control subjects.

The aim of this study was to establish nutrient intake from breakfast in children and adolescents in Croatia (n = 1190). A quantified FFQ method was used. The results showed that 1.7% of subjects skip breakfast. Energy intake from breakfast was 26% RDA. Adolescents obtained significantly more energy from carbohydrates and less from fat than children. Children had significantly higher cholesterol and significantly lower dietary fiber intake than adolescents. Intakes of almost an examined micronutrients differed significantly according to age. Milk and dairy products were major energy contributors in children's breakfast whereas most of the adolescents' energy came from cereal products. Breakfasts containing at least one dairy food, one cereal food and fruit/fruit juice were consumed by 13.7% and 21.9% of children and adolescents respectively. A total of 33.3% and 47.6% of subjects respectively consumed milk every day while 15.9% and 10.4% did not consume milk for breakfast at all. Subjects with BMI > 85th percentile had significantly higher energy intake while those with BMI < 25th percentile had significantly lower energy intake than those of normal weight. Education about adequate nutrition and human health and providing breakfast at schools would improve dietary habits of this population.

Two brothers and a sister suffering from homocystinuria caused by cystathionine beta-synthase deficiency and the possibilities of treatment are presented. Possible clinical variability, the necessity for early diagnosis and the importance of hyperhomocysteinemia as a risk factor for early-onset vascular disease are discussed.

Strict gluten-free diet, the only treatment for patients with celiac disease, is necessary to prevent malabsorption and to reduce the risk of complications. Such diet excludes all food and beverages that contain gluten and therefore may result in a nutritionally unbalanced diet. Mediterranean diet is a synonym for healthy and balanced diet. It has been shown that greater adherence to Mediterranean diet is correlated with reduced mortality. Adherence to Mediterranean diet can easily be determined using Mediterranean dietary quality index (MDQI). MDQI is consisted of seven components (saturated fatty acid, cholesterol, meats, olive oil, fish, cereals, fruits + vegetables), whereas each component depending on intake may achieve 0 to 2 points. Total index score might be between 0 and 14 points, where 0 points indicate great adherence to Mediterranean diet. The aim of this study was to evaluate the quality of a gluten-free diet based on a MDQI in Croatian celiac patients. MDQI was calculated based on a seven-day food record from 23 celiac patients from Mediterranean region and 38 celiac patients from continental region. Statistical difference was observed only for saturated fatty acid intake among observed regions (p